乳児期に手術介入を要したmid-aortic syndromeの1例

Mid-aortic Syndrome Requiring Surgical Intervention during Infancy；Report of a Case

はじめに　Mid-aortic syndrome（MAS）は胸部大動脈の遠位から腹部大動脈にかけて狭窄を認める非常にまれな疾患であるが，高血圧などを契機に発見され，幼児期～青年期に手術介入となる報告が多い．われわれは早期から著明な左室心筋肥厚を認めたMASに対し，乳児期に手術介入した．術式は人工血管をパッチとして用いたパッチ拡大術を選択し，術後圧較差も改善し，良好な結果を得たので報告する．

Mid-aortic syndrome （MAS） is a very rare disease characterized by stenosis from the distal of the thoracic aorta to the abdominal aorta, in many case it is found as a result of hypertension and the like, and it needs surgical intervention in early childhood to adolescence. Here, we report a case of MAS which recognized prominent left ventricular myocardial hypertrophy from the early stage and needed surgical intervention in the infancy. We selected patch angioplasty using expanded polytetrafluoroethylene （ePTFE） graft, and after surgery pressure gradient was disappeared.